L20 - Restrictive Thoracic Disorders Flashcards
Definition of restrictive thoracic disorders
- Lung volumes (TLC) is (reduced/increased/preserved)
- Airway resistance and expiratory air flow are (reduced/increased/preserved)
- How is TLC and RV measured?
- reduced
- preserved (normal FEV1)
- Whole body plethysmograph (aka body box) or helium gas dilution
List the 4 general categories of restrictive thoracic disorders
- Pleural
- Interstitial
- Neuromuscular
- Thoracic/Extrathoracic
Pleural effusions, pneumothorax, asbestosis disrupt mechanical function that leads to shortness of breath +/- pain are examples of what category of restrictive thoracic disorders?
Pleural
Idiopathic pulmonary fibrosis, Vasculitis, Sarcoidosis, Asbestosis are examples of what category of restrictive thoracic disorders?
Interstitial
Myasthenia Gravis, Myopathy, ALS are examples of what category of restrictive thoracic disorders?
Neuromuscular
Obesity, Kyphoscoliosis, Massive ascites are examples of what category of restrictive thoracic disorders?
Thoracic/Extrathoracic
- This structure is a continuous mesothelial surface enclosing a potential space that is 10-20 µm in width
- Parietal blood supply is from ________
- Visceral blood supply is from _______
- Innervation is from parietal pain fibers in the costal surface via ________ and diaphragmatic surface via __________
- Pleura
- intercostal aa
- bronchial aa
- intercostal nn and phrenic nn
Which nerve can result in referred pleuritic pain to chest wall and shoulder?
Phrenic nerve
T/F visceral pleura has pain fibers
FALSE - no pain fibers
Which is FALSE regarding pleural physiology?
A. Normal pleural space has greater than 5 ml of fluid
B. ~500 ml of fluid secreted and drained daily through lymphatics
C. Pleural pressure negative at functional residual capacity
• -5 cm H20 at FRC
• more negative on inspiration
• positive with active exhalation (exhalation usually passive)
A. Normal pleural space has less than 5 ml of fluid
volume of air present in the lungs at the end of passive exhalation
FRC (functional residual capacity)
- (Transudation/Exudation) is when pleural fluid accumulates due to changes in hydrostatic forces (Starling forces)
- There is (decreased/increased) capillary filtration pressure due to vol overload (renal failure) or LA HTN (CHF)
- There is (decreased/increased) plasma oncotic pressure) - due to cirrhosis or nephrotic syndrome
- Transudation
- increased
- decreased
- (Transudation/Exudation) is when pleural fluid accumulates due to increased leak across capillaries
- List the 3 “I’s” associated with this process
- Exudation
2. infection (pneumonia); inflammation (RA, SLE); infiltration (metastatic cancer)
- (increased/decreased) flow through lymphatic stroma can cause pleural fluid accumulation
- List the 3 “I’s” associated with this process
- decreased
2. infectious debris (pneumonia, empyema); inflammation (radiation fibrosis, old injury); infiltration or tumor
The most likely cause of pleural fluid accumulation is a combination of (increased/decreased) fluid influx and (increased/decreased) lymphatic clearance
- increased
2. decreased
Pleural Effusion Transudation vs. Exudation
• increased filtration pressures and/or decreased oncotic pressures
• protein poor (not actively secreted fluid), low cell counts
Transudate
Pleural Effusion Transudation vs. Exudation
• increased capillary leak and/or decreased lymphatic clearance
• protein rich, cellular
Exudates
3 Symptoms of pleural effusion
- Dyspnea
- Chest pain (if parietal pleura inflamed)
- Asymptomatic
Pleural Effusion exam findings (3)
- Percussion dullness (if large)
- Absent breath sounds
- Egophony at top of effusion
A. CXR of pleural effusions show what 2 main features?
B. What do you see on lateral decubitus view?
A
1. loss of phrenic angles (high hemidiaphragm)
2. density with meniscus
B. free flowing effusion layers
Which is false regarding thoracentesis?
A. Aspiration of fluid from pleural space
B. Indicated in all new infusions except very small effusions or CHF effusions
C. Risks: bleeding, infection, pneumothorax
D. Avoid neurovascular bundle under rib
• So needle INFERIOR to the rib
D. Put needle superior to the rib
Pleural Effusion LIGHT’s Criteria defines an exudate as any one of the following:
- Ratio of pleural fluid /serum total protein of greater than _______
- Ratio of pleural fluid /serum LDH of greater than ______
- Pleural fluid LDH greater than ____ upper limit of normal serum LDH
1.greater than 0.5
2. greater than 0.6
3. greater than 2/3 upper limit of normal serum LDH
• If none of these criteria are met, then it’s a transudate
Requires no further invasive evaluation
Treat underlying disease (e.g. diuretics, dialysis, etc)
Which has a more extensive ddx, transudative or exudative pleural effusion?
Exudative
infections, neoplasm, PE, connective tissue disorders, post cardiac injury (dressler’s synd), asbestos-related effusions, GI disorders, uremic pleuritis, Meig’s syndrome, Yellow-nail syndrome, chylothorax, trapped lung
What is this condition? • Symptoms: • Sudden onset Chest pain • Dyspnea • Exam: • Often none • Percussion hyper-resonance • Absent breath sounds • X-ray: • Hyperlucency • Loss of lung markings • Peripheral pleural line
Pneumothorax Air in the pleural space
Primary or Secondary Spontaneous Pneumothorax?
1. no underlying lung disease
2.Rupture of apical subpleural blebs (seen on CT)
3.High incidence in smokers and tall, thin habitus.
4. High rate of recurrence
○ 50% after first; 65% after second; 80% after third.
Primary
Primary or Secondary Spontaneous Pneumothorax?
- pre-existing lung disease - COPD, cystic fibrosis, tuberculosis etc
- Catamenial Pneumothorax - associated with menstrual cycle - pleural endometriosis vs transdiaphragmatic passage of peritoneal air.
- Treat with suppression of ovulation or pleurodesis
Secondary
Traumatic or tension non-spontaneous pneumothorax?
1. Penetrating injury (stab wound)
2. Sudden increase in transpulmonary pressures (mechanical ventilation, blunt trauma, decompression sickness)
○ e.g. patient with severe ARDS
3. Iatrogenic - mechanical ventilation, lung biopsy, central lines
Traumatic
Traumatic or tension non-spontaneous pneumothorax?
- severe dyspnea, hypoxemia, cardiovascular collapse - absent breath sounds
- Suspect when sudden deterioration on ventilator, after biopsies/line placement
- Treatment - 100% oxygen and immediate pleural decompression
- DO NOT wait for x-ray confirmation
tension
Describe the following Pneumothorax Managements
- Observation
- Oxygen
- Needle aspiration
- Tube thoracostomy
- Pleurodesis (Sclerosing agents,Surgical pleurodesis)
- Observation
• for small (
Primary Neoplasm of Pleura or Secondary metastasis to pleura?
- very rare
- Mesothelioma, Sarcoma, Neuroma
Primary Neoplasm of Pleura
Primary Neoplasm of Pleura or Secondary metastasis to pleura?
- more common
- Lung, breast, and lymphoid cancers account for 75% of all malignant effusions
Secondary metastasis to pleura
A.Cytology of fluid positive in 40-60%, increased yield with repeat taps
B. Treatment (similar to pneumothorax):
○ Observation - if small effusion
○ Large volume thoracentesis - Indwelling catheters allow for home therapy
○ Pleurodesis - chest tube, or thoracoscopic
What is this condition?
1. Asbestos exposure:
• 50% of cases have clear exposure history (latency of >20 years)
• Increased risk with exposure to other silicates, thoracic irradiation, coal etc
2. Symptoms - chest pain and dyspnea, fever, fatigue
3. Signs: 50% have an exudative effusion (bloody)
4. Diagnosis - pleural biopsy
5. Therapy - palliation of pain and dyspnea; Little response to most multimodality treatments
Malignant Mesothelioma
Which is NOT a cause of interstitial lung disease?
A. Idiopathic: idiopathic pulmonary fibrosis (IPF)
B. Autoimmune: rheumatoid arthritis, Wegener’s granulomatosis or scleroderma
C. Malignancy - mesothelioma
D. Hypersensitivity Pneumonitis: Mold, organic dust
E. Post infectious: bronchiolitis obliterans with organizing pneumonia (BOOP)
F. Occupational exposures
• Silicosis: foundry workers, sand blasters
• Asbestosis: pipe fitters, shipyard workers
G. Medications: Methotrexate, cyclophosphamide
C. this is related to pleural diseases
In interstitial lung disease, the lungs become stiff (in contrast to emphysema). This is due to:
- Increase/decrease in compliance decreases
- Increase/decrease in elasticity
- compliance/elasticity is a change in volume/change in pressure
- decrease
- increase
- compliance
Which restrictive thoracic disorder has the following symptoms?
1. Nonspecific
• Shortness of breath, insidious onset
• Dry cough, little to no sputum production, unless bronchiectasis is present
• No fevers or chills
• Symptoms of associated disorders such as autoimmune
A. Pleural
B. Interstitial
C. Neuromuscular
D. Thoracic/extrathoracic
B Interstitial
Which is not a sign of interstitial lung disease
A. absent breath sounds
B. Clubbing, cyanosis or rash
C. Skeletal deformities if associated with autoimmune diseases (rheumatoid arthritis)
D. Advanced stages: Evidence of pulmonary hypertension (loud P2, RV heave) - cor pulmonale
A. Rales on lung exam, heard best at the bases
Which is false regarding the lab findings of interstitial lung disease
A. Elevated erythrocyte sedimentation rate and other acute phase reactants
B. Elevation of autoantibodies
• Disease specific (rheumatoid factor and anti-nuclear antibody)
• Nonspecific (anti-neutrophil cytoplasmic antibody)
C. Anemia of chronic disease or polycythemia
B• Elevation of autoantibodies
• Nonspecific (rheumatoid factor and anti-nuclear antibody)
Disease specific (anti-neutrophil cytoplasmic antibody)
PFT of COPD
- obstructive/restrictive physiology
- low ____ compared to ______
- high/low FEV1/FVC ratio
- high/low lung volumes
- high/low diffusing capacity
- high/low pCO2 level
COPD
- Obstructive physiology
- Low FEV1% compared to FVC%,
- low FEV1/FVC ratio
- High lung volumes
- Low diffusing capacity
- May have high pCO2
PFT of interstitial lung disease
- obstructive/restrictive physiology
- low ____ compared to ______
- high/low FEV1/FVC ratio
- high/low lung volumes
- high/low diffusing capacity
- high/low pCO2 level
Interstitial lung disease
- Restrictive physiology
- Low FVC% compared to FEV1%,
- High FEV1/FVC ratio
- Low lung volumes -Don’t have as much trouble exhaling
- Low diffusing capacity
- Often low pCO2 until end stage
ILD on CXR shows decreased lung volume and change of scarring.
If the patient has IPF, RA associated disease, scleroderma and general CT diseases, scarring is seen where?
Lower lobes
ILD on CXR shows decreased lung volume and change of scarring.
If the patient has sarcoidosis, silicosis (inhalation and occupational illnesses) scarring is seen where?
Upper lobes
ILD on CXR shows decreased lung volume and change of scarring.
If the patient has drugs, asbestosis as cause of ILD, scarring is seen where?
diffuse (no specific directions)
ILD on CXR shows decreased lung volume and change of scarring.
If the patient has Wegeners granulomatosis, what type of scarring is present?
Cavitary
ILD on CXR shows decreased lung volume and change of scarring.
If the patient has bronchiolitis obliterans with organizing pneumonia
Patchy (ground glass)
Why is chest CT for ILD beneficial?
- More definition of the underlying lung parenchyma
- Fibrosis – thickening of the interstitial markings in the lung
- Ground glass – hazy areas of increased density in the lung
- Honeycombing – areas of the lung that have fibrosis and a pattern suggestive of a honeycomb
collections of cells in a whorl pattern, suggests sarcoidosis or foreign body exposure
Granulomas
silica or talc are birefringent under a polarizing microscope, asbestos bodies look like a brown dumbbell
Foreign bodies
Which is false regarding idiopathic Pulmonary Fibrosis
A. Etiology unclear (5% familial)
B. Occurs in older (6th-7th decade) people – Familial cases occur at a younger age
C. Male predominance
D. Smokers have more severe disease
E. Diagnosis of exclusion- cannot be made in the setting of autoimmune disease or environmental exposure
F. CXR/CT shows upper lobe predominance
G. 50% survival at 3 years
H. Generally unresponsive to therapy – Newly FDA approved: Pirfenidone, Nintedanib
F. basal predominance
IPF is an interstitial lung disease
Which is false regarding collagen vascular disease?
A. ILD may present as the last sign of CVD
B. May be associated with therapy used (methotrexate)
C. Autoimmune: Rheumatoid arthritis, Scleroderma and Sjogrens are the most common
D. Better prognosis than IPF: Course tends to be indolent as compared to IPF, sometimes responds to therapy
A. First
What is this condition?
• Unknown etiology
• Mostly asymptomatic – Often recognized on routine CXR with hilar adenopathy or because of eye complaints (uveitis)
• May have a rash preceding symptoms – (E. nodosum)
• Upper lobe predominance on imaging, hilar lymphadenopathy, nodules
• Pathology: granulomas (pathomneumonic)
• Good response to steroids, – but treatment not always needed
Sarcoidosis - Interstitial lung disease
What environmental ILD is this?
- Requires significant exposure (years)
- Upper lobe predominant because of inhalational properties - Egg shell calcification of lymph nodes
- Pathology: birefringent particles
- Sandblasting, foundry work
Silicosis
What environmental ILD is this?
- Pleural and parenchymal disease
- Pipe fitters, shipyard workers
- 15-20 years latency
- Pathology: ______ bodies
- Increased risk of malignancy in smokers
- CXR: calcified pleural plaques
Asbestos exposure
4. asbestos
What ILD is this?
- May present with hemoptysis
- Antineutrophil cytoplasmic antibodies present in cytoplasmic pattern (classic; c-ANCA; PR3-ANCA; 80-90%) - autoantibodies
- Often cavitary disease on radiography
- Very responsive to immunosuppressive agents, but does relapse
Wegener’s Granulomatosis
What ILD is this?
- Frequently occurs after a respiratory infection
- Radiography shows patchy areas of ground glass opacities
- Pathology shows plugs of fibrotic material in the terminal bronchioles with distal pneumonic process
- Responsive to steroids
Bronchiolitis Obliterans with Organizing Pneumonia
marked by lung scarring with decreased lung volumes and compliance
Interstitial lung diseases
Most common initial treatment is corticosteroids
Interstitial lung diseases
Which is TRUE regarding neuromuscular Disorders
A. Weak respiratory muscles do not generate sufficient positive inspiratory pressure for full inhalation
○can cause basal atelectasis; Impaired cough and aspiration risk, recurrent infections
B. Diaphragmatic weakness: Paradoxical outward movement of abdomen with inspiration
C. Sleep disordered breathing: slowly decreases PCO2
D. Low FVC, Low NIF (negative inspiratory force)
E. Requires invasive procedures to reduce symptoms
D.
A. Weak respiratory muscles do not generate sufficient NEGATIVE inspiratory pressure for full inhalation
B. Diaphragmatic weakness: Paradoxical inward movement of abdomen with inspiration
C. Sleep disordered breathing: slowly elevates PCO2
E. Supportive care: oxygen, assisted cough, noninvasive ventilation (when they get hypercapnea), tracheostomy to manage secretions
Complete or partial collapse of a lung or a section (lobe) of a lung
Basal atelectasis
Kyphoscoliosis
Excessive AP (________) and lateral (_____) curvature of the thoracic spine
• Excessive AP (kyphosis) and lateral (scoliosis) curvature of the thoracic spine
Which is False regarding kyphoscoliosis?
A. 3% of those with abnormal curvature have symptoms
B. Idiopathic, Congenital (Muscular dystrophy or ataxia) or Neuromuscular (poliomyelitis - get after polio)
C. Restrictive PFTs, Hypoventilation, Pulmonary hypertension
D Degree of scoliosis correlates with ventilatory impairment – Cobb Angle > 90
E. Surgical correction, nocturnal ventilation
F. Decreased FRC, mechanical disadvantage of the deformity causes increased work of breathing, High RV,
G. Low TLC and VC.
G. Low TLC and increased VC
